Children with Auditory Neuropathy Spectrum Disorder present a challenge for early intervention and habilitation because of the diversity of the disorder and the current lack of robust indicators to guide in management of this population. These challenges can result in significant delays in selecting and implementing appropriate interventions such as hearing aids and/or cochlear implants. This project focuses on cortical auditory evoked potentials as a class of objective electrophysiological measures that hold great promise in the prediction of intervention outcomes. The proposed work evaluates the association between these potentials and speech perception in children with this disorder by pursuing two specific aims. Aim 1 focuses on the sub- group of children who use hearing aids and evaluates the utility of acoustically-evoked cortical potentials for predicting success with these devices. Aim 2 focuses on the sub-group of children who use cochlear implants and evaluates the relationship between electrically-evoked cortical potentials and speech perception. Cortical potentials (P1-N1-P2 complex and the change complex) will be recorded from each child in response to either acoustical or electrical stimulation, as appropriate for their device. In addition, aided speech perception performance will be measured using age- and developmentally-appropriate tests. Correlations between the electrophysiological measures and speech perception performance will then be assessed. The goal of these experiments is to identify electrophysiological response features that are most informative in terms of guiding intervention strategy. As such, this project has tremendous translational significance because its findings are likely to directly impact clinical practice. PUBLIC HEALTH RELEVANCE: Experiments are proposed to evaluate the relationship between the electrophysiological measures of cortical evoked potentials and the speech perception performance in children with auditory neuropathy spectrum disorder (ANSD). The ultimate goal of the proposed study is to develop tools for early and accurate identification of the best rehabilitative strategy for each individual patient. It is likely that the electrophysiological measures investigated in this program of research might be adopted as part of clinical test-battery for ANSD patients, which will have an important impact on clinical practice.